Mamede de Carvalho – good humour at the service of life

When we hear him talk, we wouldn’t tell that he was born in Rio de Janeiro and only later moved to Brasília, where he lived until he was 13, living in Lisbon between ages 7 and 10, before returning to Portugal for good. So many years later there is no trace of the accent from Rio de Janeiro, however, a few typical expressions give him away. His father, a Chef in two of the best hotels in Brazil, was in charge of serving some of the best meals to important historic governmental figures of the world and Brazil: Henry Kissinger, Jimmy Carter, Eduardo Frei, Ernesto Geisel, Costa e Silva, Garrastazu Médici, Américo Thomaz are just some of them.

He remembers being a child and walking across the long blocks of inhospitable and dry Brasilia, with its reddish soil, looking for a “bar” where he could drink a glass of guarana. Contrast of two worlds that may explain part of his personality.

Mamede Alves de Carvalho doesn’t have double nationality, he’s only Portuguese, but there are still traces of his Brazilian origins in the way he dances samba with life and sees its bright side without the tragic Lusitanian mark.

Deputy Director of the Faculty of Medicine of the University of Lisbon, a position entrusted to him by the Director, he is, quintessentially, a Neurologist at the Lisboa Norte Hospital Centre.Group Leader at the Institute of Molecular Medicine (IMM-João Lobo Antunes), he is also the Director of the Institute of Physiology. Specialised in neuromuscular diseases and electromyography, in particular ELA – Amyotrophic Lateral Sclerosis – he deals, on a daily basis, with patients with the most severe degenerative disease of the nervous system, which leads to death due to respiratory and motor insufficiency in 3 to 5 years.

One of few Europeans awarded with the Forbes Norris prize, which distinguished a physician who shows exceptional care and compassion while studying and researching this disease.

Despite having an interest in Physics, he says he chose Medicine without much hesitations due to a “childhood impulse”. During his two-year internship he focused on General Medicine, and later he went to the Cascais Hospital where, according to him, he worked rather hard. He probably doesn’t remember most of his New Year’s Eves, but there is a specific one he mentions off the top of his head. A group of people ended up in the emergency room, some of them in a very critical condition, due to a severe traffic accident. He made several decisions having no time to hesitate, just like he had to believe in himself, saving lives of his patients that night. After sewing and identifying vertebral-medullary lesions, sending some patients to the central hospital, he realised that nothing had gone wrong and that he had passed his first major test. On other occasions, which will not happen again, he performed several births and C-sections. Finally, when the time came to choose between Neurology and Neuroradiology, he chose the former. In 1988 he did an internship in Neurology at Santa Maria, recognised as the best Hospital to learn and practise Neurology. “Egas Moniz’s legacy persisted in my time and it still does because there was a curiosity and will to study and publish new things”.

Then he met Professor Sales Luís, his mentor; she specialised in neuromuscular diseases, and was also the Director of Neurology Department. He came into contact with Neuromuscular Pathology and Electromyography early on, becoming one of few young Neurologists with a subspecialisation in Neuromuscular diseases. In this world of diseases with a wide variety of derivations, he realised that there were two he found particularly challenging, Paramyloidosis (a disease with an impact on the northern coast of Portugal, but is also significantly prevalent in Lisbon), and Amyotrophic Lateral Sclerosis (ELA). Since his mentor, Sales Luís, was responsible for creating the first appointment in the country dedicated to ELA and one of the pioneers at the European level, Mamede de Carvalho soon started following both appointments intensively. For many years he was the sole responsible for the Paramyloidosis appointments and, at the time, he followed only a few patients in the ELA appointments. “While in the past I had 4 appointments per week, today I see on average 20 patients and if I had more time, I would see about 50”. This is a complex issue that doesn’t have only one obvious answer, nevertheless, but the most likely explanation is that diagnosis is being done at an increasingly earlier age and people have increased their life span. Usually, the disease beings after age 60, despite sometimes being triggered in young patients, and it is predominant in males. Once he receives a patient Mamede de Carvalho doesn’t let him go, contradicting the medical records of other countries, such as England, where, in a more advanced phase, patients end up receiving only palliative care.

With more than 300 published articles, he studies behind the scenes and also works with his patients on daily basis. There is no difference between working days, holidays, or weekends, as being an international reviewer of scientific works, he reads and reviews dissertations and projects from his peers. In 2018 he received the Publons Peer Review Award, standing out as one of the most productive international reviewers.

He jokes that he only has 4 cats, the maximum according to regulations, but in reality, he as 7, all rescued from the streets. One of them, Óscar, followed him to his house and never left, neither his owner nor the couch. He looks at animals as if they were humans, “they are nice, they let me live there”. He has fun choosing names for his pets – once he had a dog named Rebeca- and he admits that his originality extends to his students, as he writes pseudonyms in exams that, despite being imaginary, always reveal something about the clinical case presented in the question.

Mamede de Carvalho doesn’t always joke, far from that, he keeps his humour for very specific situations, when he trusts who he is talking to, or when faced with certain situations. There are issues that take away the lively look of the boy from Rio de Janeiro, the constant anxiety due to the change of politicians and their policies, the conditions of Healthcare and the lack of funding in education, or the disease he deals with every day, Amyotrophic Lateral Sclerosis. From the beginning of a conversation to the analysis of the status quo, to the beginning of life, when apparently it has a predefined expiration date. These were our basic points.

The picture of this disease is drastic. Patients who suffer from ELA die in a few years, after a devastating suffering, losing all motor and respiratory capacities. Do you ever feel frustrated facing an enemy called ELA that keeps on beating you and challenging you?

Mamede de Carvalho: Only a person who has an enormous capacity to deal with frustration can follow this type of patients. And I do have it. Of course it is frustrating, but in life we need to have the capacity to cope with that frustration. I know that the patient in front of me is suffering and that he will die, but I also know that if nobody is there to support him, it will be even worse. Therefore, my obligation is to understand him, to empathise with him in order to help him as much as possible. I have to support that patient leaving my own interpretations of life out of it; in order to help him I cannot think about the frustrations it may cause me. Of course it is always difficult to deal with this. But, you know, I think that the old way makes no sense, sending patients home, telling them that the disease is progressing and leaving them alone from the clinical point of view. That is not reasonable. When we give our support, we always need to keep hoping to see a light at the end of the tunnel. Also, there are different types of patients, more sophisticated ones who are informed about everything, and others who didn’t look anything up and don’t wish to do so. You need to moderate the hope factor according to each patient’s language, explaining that there have been advances in research.

Is that hope real? Because there has been a fight for proper research in these fields in which you are also involved.

Mamede de Carvalho: Absolutely, but we cannot be personal when talking about different researches, we need to have a more universal approach. There are indeed many brilliant researchers all over the world studying this disease, because it is a terrible challenge.

What is it that you look for in developed researches, exactly?

Mamede de Carvalho: First of all, to understand the disease, because we are not very familiar with the processes of the cell death mechanism, we know little about the initial factor that triggers cell death. And then we need to understand how the disease spreads. Another important fact is that more than 30 genes with different actions and properties are involved in the disease and it is important to find out what they have in common and what could justify the fact that patients with no mutations may share the primary mechanism that may lead to cell death. There is a lot of research left to do in this area. Many researchers are also doctors so they have a basis of clinical integration and they are particularly concerned and attentive when it comes to looking for an answer for their patients. We are trying to identify molecular mechanisms that could allow us to develop drugs to fight or slow down the evolution of the disease. Now these aspects are motivating for patients.

What is the focus of your research?

Mamede de Carvalho: My most historical dedication has been neurophysiological research (including diagnostic criteria), as well as topics regarding ventilatory dysfunction, respiratory support and methods to evaluate the progress of involvement of the respiratory muscles. My most recent focuses examine molecular biomarkers, genetic studies (in collaboration with international consortia), phenotypes, as well as neuromodulation via percutaneous electrical stimulation.

Is there a way to anticipate this disease?

Mamede de Carvalho: No, there are early clinical manifestations like the lack of strength and electrophysiologic manifestations like the loss of motor units, but we are not able to do screening to anticipate diagnosis or intervention. About 1 person in 12000 will develop this disease.

Many people comment on your very sophisticated sense of humour. Can you tell me how you stay balanced in your everyday work?

Mamede de Carvalho: I always had a sense of humour. And my personality is not contaminated, it is not changed, by the patients I follow. Patients don’t want to see me depressed either. On the other hand, we have to keep a certain distance from our own suffering, but you know, I laugh a lot with my patients? Most of them want me to be that way, people who don’t suffer from any disease are actually the ones who don’t like it. (Laughs)

Is there a lot of truth in your humour?

Mamede de Carvalho: Absolutely. It is a good weapon, isn’t it?

Yes, especially because not everybody understands it.

Mamede de Carvalho: Exactly. (Laughs loudly)

We wanted to discuss with you your views on different beginnings, this months’ topic. As deputy director of the Faculty and as a doctor. As a doctor and now that a new year has begun, what are your views on the over-indebted National Health Service?

Mamede de Carvalho: The issue with Healthcare is that it is very complex, because it requires a planned and sound administration, something that is not often seen in Portugal. The country doesn’t make plans in advance, sometimes it does because it needs an emergency solution, and the decisions that are made are often changed by the following ministers, sometimes from the same government. And this is absurd because there should be consensual, rational and structured programs that are firmly respected to the end. That is the way in Science. Once a researcher plans his tasks, he doesn’t change them halfway through. He respects and acts in accordance with the scientific rules. The same goes for an employee in a company, he has to respect the existing strategic decisions. In Healthcare we miss those well-established action lines. There is also another problem, healthcare professionals are underpaid and they have created unreal, but fair expectations with the supposed end of the crisis. Unreal expectations are the worst, also for patients because it creates a lot of confusion. On the other hand, Healthcare is becoming very expensive and innovative treatments have very high costs. I am not referring only to cancer, but also to neurological diseases, such as multiple sclerosis. Although there are general rules on drug use, it is often hard to manage the cost of drugs due to the lack of a strict control of their use. As for 2019, it will be a transition year, given the fact that it is an election year, the decisions will seek popularity, but there should be clear lines with regard to the National Health Service. In particular, it is urgent to make decisions to make it clear whether the Country wants a strong and efficient National Health Service that will meet the population’s needs; or whether they want to support private-based Healthcare. I think that the first option will win, but it requires a higher investment. You know, it always surprised me that such a small and poor country like ours could have more private healthcare units than a rich Northern-European country. In Germany, France and other countries, there are still old private practices, with the doctors’ names on the door, and here, these intimate practices disappeared; in every small town or neighbourhood, I only see units from large private groups. Today even private hospitals can train interns, which is debatable. Does it make sense that private units train doctors? Do they have the necessary competences and independence from the economic point of view (in the strange patient/user/client relation)? The country needs to think very carefully about the model it wants to adopt.

We have another matter of concern on the table, the possibility to remove tuition fees. How does our Faculty see this matter?

Mamede de Carvalho: With concern. It raises very disturbing questions. Personally, I think that eliminating tuition fees in Portugal means that the poorest population will strongly contribute to the studies of the most favoured population through their taxes. Furthermore, as statistics show that higher education is associated with a higher salary, tuition fees result in a small effort of individual investment in oneself for future gains, economic return, it is close to the philosophy of any other investment in the financial market. Of course, disadvantaged students always need to have strong support so that their studies are not compromised due to financial constraints. From the institutional point of view, once the government stops providing compensation for the amount universities lose due to lower tuition fees, that decision tends to create an unsustainable economic bottleneck situation in this and other Portuguese universities. Since the famous crisis, which severely affected higher education, universities are chronically underfunded, an additional difficulty is a high-risk exercise. So, it seems to me that it is a paradox to want, on the one hand, a high percentage of the young population taking advantage of higher education and high differentiation and universities with good research and productivity at the international level, and at the same time make successive funding reductions. Either they are regarded as important and get funding. Or they are not so important after all. Decisions have to make sense. When we had the major bank crisis in Portugal, leaving these banks out of the State budget was seen as a high risk, due to the possible domino effect on the economy. Now, if universities are important, they should follow the same line of reasoning. I think that paying a tuition fee, which is already symbolic, is acknowledging that everything has a price. From the psychological point of view, people tend to underestimate things they don’t have to pay for. This tuition fee for me means that students acknowledge the effort society puts into their education. However, the support mechanisms for those who really need help in order study need to be strengthened.

Is being close to your students important to you? Does it help you to stay closer to reality?

Mamede de Carvalho: That proximity to young people always has a very refreshing effect. (Laughs) Especially for those who are not so young any more, it gives us biological advantage. But I also think that younger people benefit from those who can give them information or guide them based on their experience.

Is that the definition of a Professor and his role, “sharing experience and wisdom”?

Mamede de Carvalho: Today, knowledge has lost its importance because you can find it on youtube. In the past it had a great deal of importance because it was only in the minds and books of illustrious Professors.

Do young people have this latent disdain with regard to the knowledge of the elderly?

Mamede de Carvalho: I wouldn’t call it disdain, I would say that now there is the perception that knowledge is easy to obtain. In my days we had to save a lot to buy a book. Now you have everything online. Now, there is another aspect as important as knowledge, it is guidance and capacity, especially for a doctor, to put the acquired knowledge into practice. In our classes we explore the knowledge that is useful for a doctor regarding his role in his patient’s life. We are here for that, to provide guidance in case of doubts in education and professional career and, in this context, the experience of the elderly is very important and usually very well received.

António Cavaco Silva, brother of the former President of the Republic was one of his patients and whose illness wanted to make it public

Professor, can you tell us how one looks at life after being diagnosed with a severe disease? How do we move on when we lose our health, can you tell us?

Mamede de Carvalho: Well… there are two different scenarios. A young person who deals with severe disease and an elderly person. People who are 70/80 years old and always had an independent and active live, have their own vision of dignity. They have lived, they already know what life is like, they have gained a lot of experience, and some of them are already a bit tired. They, generally, don’t accept to live without their dignity, they prefer to die when the time comes. And they are saying it sincerely. And I understand this very well. Because they cannot dress themselves, wash themselves and that is a humiliating dependence. It’s different with young people, and I have some with ELA who are only 30/40 years old, they want to live and they adapt to almost anything. They believe there is still a way, despite suffering from a severe disease. You know I always talk to my patients’ relatives, those who come, and I always mention this future loss of dignity, especially in case of the elderly who were always healthy, and that death is inevitable. There are many who think that it is a blessing to die as soon as possible. We need to respect that, not respecting the patient’s point of view is a huge mistake of the society.

And what can you do in those cases as a doctor?

Mamede de Carvalho: I can only help them to cope with pain, helping them to maintain their dignity as much as possible. Always having in mind my position as a doctor and all the rules I have agreed to follow. But I can show empathy and say that I would feel the same. Dignity is the most important expression of any individual.

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Still without a cure, ELA is now manageable with medication and ventilatory support. The rest, which is still not enough, is built in the doctor-patient relationship.

President of the scientific board of the APELA – Portuguese Association of Amyotrophic Lateral Sclerosis – Mamede de Carvalho, manages the ELA centre at the CAML and he does research on other ways of prolonging life. In touch with other centres around the world, he is always trying to find new solutions and ways to comfort patients.

When he told me about his life with his cats and books, I asked him if he felt lonely. I realised that he doesn’t, not at all. He’s totally surrounded by life and knowledge, but he never complains about the difficulties he is faced with on a daily basis. Partly because, as he said, “life without humour is not funny at all”.